Special Health Care Needs Flashcards

Question

Recommendation for permanent tooth endo tx prior to cancer tx?

Answer 1

Symptomatic, non-vital permanent tth: do RCT \> 1 wk before chemo bc need time to assess success of RCT Asymptomatic, non-vital perm tth RCT may be delayed until hematological status of pt is stable. Otherwise, EXT EXT followed by abx for 1 wk if RCT can’t be completed in single visit \*\*determine etiology of PARL associated with RCT-txed teeth to diagnose pulpal infection, inflammatory rxn, apical scar, cyst, malignancy. If PARL associated with RCT tooth & no s/s infection, could be apical scar (no need for retreat or ext)

Answer 2

If OH poor, tx protocol or HCT conditioning has moderate to high risk of developing mucositis, remove appliances. If can’t remove, use wax/mouth guard to decrease tissue trauma If poorly fitting, remove bc can abrade mucosa and increase risk of microbial invasion of deeper tissues Simple things like B&L, LLHA that are not irritating may be left in place for pts with good OH. Removable appliances may be worn as tolerated. Clean them with antimicrobial solutions

Answer 3

Operculum should be excised if potential risk for infection d/t pericoronitis & if hematologic status allows. Perio assessment needed prior to bisphosphonate therapy part of cancer tx EXT for tth with poor prognosis that can’t be treated definitively w/perio therapy

Answer 4

Allow them to exfoliate naturally

Answer 5

Ideally 2 weeks At least 7-10 days allow adequate healing

Answer 6

No, we discourage them because they do not allow for effective cleaning – only use if pt develops moderate to severe mucositis Toothpicks and water irrigation devices should not be used when pt is pancytopenic (avoid tissue trauma)

Answer 7

mucositis salivary effects- reduced flow, thicker, lower pH opportunistic infx (fungal, viral, bacterial) oral bleeding: thrombocytopenia, others soft tissue necrosis dental sensitivity/pain: salivary changes, neurotoxic drugs, odontogenic infx, referred pain taste dysfunction mucosal and muscular fibrosis trismus, TMJ dysfunction nutritional deficiencies GVHD in HSCT craniofacial, dental development problems

Answer 8

No. Recommend good OH, analgesics, non-medicated rinses like 0.9% saline or sodium bicarb rinse 4-6xday, parenteral nutrition as needed

Answer 9

Palifermin is keratinocyte growth factor-1Recently recommended for prevention of oral mucositis as prophylaxis and tx to decrease mucositis Observed to decrease incidence and duration of severe oral mucositis in pts undergoing conditioning w/high dose chemo with or without radiotherapy, followed by HCT (hematopoietic cell transplant)

Answer 10

Coating agents like Amphojel Kaopectate hydroxypropylmethylcellulose Film-forming agents like Zilactin Gelclair low-level laser therapy – limited but encouraging evidence NOT RECOMMENDED: sucralfate, antimicrobial lozenges, pentoxifylline, and granulocyte-macrophage-colony stimulating factor mouthwash chlorhexidine not recommended for prevention of mucositis

Answer 11

Topical anesthetics. No significant evidence of the effectiveness or tolerability of these. Only provide short term pain relief. Lidocaine use may obtund or diminish taste and the gag reflex and/or result in a burning sensation, in addition to the possible cardiovascular and CNS effects

Answer 12

May present w/ deep, constant pain affecting mand molars in absence of odontogenic pathology. Pain usually transient & subsides shortly after dose reduction/cessation of chemo

Answer 13

No. Recommended: sugar-free chewing gum or candy, sucking tablets, special dentifrices for oral dryness, saliva substitutes, frequent water sipping, alcohol-free oral rinses, oral moisturizers, humidifier, fluoride rinses and gels

Answer 14

After completion of all therapy and after at least a 2 year disease-free survival, when the risk of relapse is decreased and pt is no longer on immunosuppressive drugs

Answer 15

Phase I: Preconditioning Phase II: Conditioning neutropenic phase Phase III: Engraftment to hematopoietic recovery Phase IV: Immune reconstitution/recovery from systemic toxicity Phase V: Long-term survival

Answer 16

1. HCT pt receives all the chemo and/or total body irradiation in just a few days before the transplant2. There will be prolonged immunosuppression following transplant.

Answer 17

Until immunologic recovery At least 100 days after HCT Longer if GVHD or other complications present \*tx should be completed before pt becomes immunocompromised

Answer 18

Starts when pt admitted to hospital to begin transplant conditioning until 30 days post HCT - mucositis (begins 7-10 days after initiation & last abt 2 wks after end of conditioning) - xerostomia - oral pain - hemorrhage - opportunistic infections - taste dysfunction - neurotoxicity (including dental pain, muscle tremors) - TMD (pain, HA, joint pain) - GVHD (with allogeneic transplant) – severe inflammation/ mucositis \*Dental tx usually not allowed in this phase due to severe immunosuppression

Answer 19

Complication severity decrease 3-4 wks after transplant Oral fungal and herpes simplex infections notable Acute GVHD xerostomia, hemorrhage, neurotox, TMD, granulomas/papillomas sometimes are observed Acute sensitivity to thermal stimuli btwn 2-4 months s/p tx w/spontaneous resolution w/in few months. Use topical F- to reduce symptoms.

Answer 20

After day 100 s/p HCT Oral complications related to chronic toxicity: - salivary dysfunction (+xerostomia) - craniofacial growth abnormalities - late viral infections - oral chronic GVHD - oral squamous cell carcinoma Mucosal bacterial infections less frequently seen, unless pt is neutropenic or with severe chronic GVHD Exam + xrays can be done. Defer invasive dental tx in profoundly immunocompromised pts.

Answer 21

\< 6yo at time of cancer therapy (\<4 y.o. especially per Flaitz) - tooth agenesis - microdontia - crown disturbances (size, shape, enamel hypoplasia, pulp chamber anomalies) - root disturbances (early apical closure, blunting, changes in shape or length) - ectopic eruption - reduced mandibular length - reduced alveolar process height - malocclusion, facial deformities, skeletal hypoplasia - trismus - may experience permanent salivary gland hypofunction/dysfunction or xerostomia - increased caries risk Relapse can develop during stage V

Answer 22

\< age 6, Especially \< 4yo - tooth agenesis - crown malformation/microdontia - enamel hypoplasia - root V shape, blunting, altered root number

Answer 23

1. BMT 2. PBSCT Peripheral blood stem cell transplantation Purpose: to restore stem cells that are destroyed by doses of chemo or radiation therapy

Answer 24

1. Autologous: pt’s own stem cells 2. Syngeneic: stems from identical twin 3. Allogenic: stems from HLA-compatible donor, related or non-related

Answer 25

Leukemia + lymphoma Also, aplastic anemia, solid tumors like neuroblastoma, inborn and acquired immune disorders

Answer 26

1. Pretransplantation phase 2. Conditioning/ neutropenic phase 3. Initial engraftment to hematopoietic reconstitution 4. Immune reconstitution/late post-transplantation

Answer 27

Conditioning / neutropenic Acute GVHD begins at this time

Answer 28

4 wks after transplantation GVHD may be present sensitivity to thermal stimuli

Answer 29

Allogenic transplant Transplanted T-lymphocytes recognize host’s cells as foreign Acute: occurs up to day 120 post-transplant - rash, GI, hepatitis - mucosal atrophy/erythema/lichenoid rxn tx: steroids/immunosuppressants Chronic GVHD: occurs btwn 70-500 days post-transplant (avg 6 months) - 20-50% of children (higher in adults) - immune dysfn or autoimmune - many organs affected: skin, liver, joints, eyes, mouth, lung, muscle - in addition to atrophy/erythema/lichenoid/xerostomia/ulcers/gingivitis/caries/perio/trismus/taste dysfunction - cyclosporine =\> gingival overgrowth

Answer 30

Coxsackie A (enterovirus) also causes hand food mouth dx 2-6 red macules, vesicles, ulcers on the soft palate, tonsillar pillars; ulcers heal in 7-10 days sore throat, dysphagia, fever, rhinorrhea, cough, vomiting, headache, diarrhea

Answer 31

Deletion of 22q11.2

Answer 32

Velocardiofacial syndrome VCFS

Answer 33

CATCH – 22 C – cardiac A – abnormal facies T – thymic hypoplasia C – cleft H – hypocalcemia 22 – chromosome 22q11.2

Answer 34

- VSD - truncus arteriosus (one vessel instead of two leaving heart)- Tetralogy of Fallot

Answer 35

1. Pulmonary stenosis (narrowing of R V outflow) 2. Overriding aorta (aorta situated above the VSD and connected to both R and L Ventricle) 3. VSD (hole btwn R & L V) 4. RV Hypertrophy (R ventricle extra muscular bc of obstruction to outflow)

Answer 36

Small, low set ears wide set eyes hooded eyes long face flattened philtrum

Answer 37

T-cells mature in the thymus. Children with small or missing thymus have poor immune function and frequent, severe infections Thymus decreases in size & function with age.

Answer 38

Cleft palate is very common (overt or submucous cleft) May cause Speech problems VPI

Answer 39

Hypoparathyroidism Parathyroid glands regulate PTH if less PTH then you have low levels of Ca and high levels of Phosphorus in the blood

Answer 40

Delayed tooth eruption but normal development; +/- enamel hypoplasia; enlarged tongue, lips (glycosaminoglycans) also FTT, short, generalized edema, puffy face, husky voice, intellectual disability mandible underdeveloped, maxilla overdeveloped

Answer 41

Lip – at 3 months Palate – at 10-12 months because negatively impacts speech development if done later

Answer 42

1:500 – 1:1000 Prevalences (AAPD COMP REVIEW) 1/2000 cleft palate alone 1/1000 cleft lip alone CL+CP 45%, CP alone 30%, CL alone 25% genetic, environmental causes (maternal alcohol use, cig smoking, folic acid deficiency, meds)

Answer 43

4-7th week of gestation

Answer 44

Boys :girls, 2:1

Answer 45

Left side more frequency

Answer 46

Cleft palate more common in girls

Answer 47

30 minutes

Answer 48

Late (8-12 hrs later) Airway inflammation Ultra-late phase (days to weeks later) Bronchial hyper-responsiveness

Answer 49

Mild intermittent/mild persistent - no night symptoms - tolerates exercise - wheezing \<2 days/wk

Answer 50

- Some night systems - Limited tolerance to exercise - Wheezing 2-5 days/wk

Answer 51

- Frequent night symptoms - Poor tolerance to exercise - wheezing every day

Answer 52

Albuterol B2 agonist (relaxes bronchial sm muscle), rescue inhaler Proventil, Ventolin Ipratroprium Atrovent – anticholinergic, blocks Ach receptors on sm muscle thus relaxes sm muscle

Answer 53

corticosteroids inhaled anti-inflammatory and immunosuppressive Fluticasone (Flovent), Budesamide (Pulmicort) Prednisolone (systemic)

Answer 54

Salmeterol, formoterolused In combo with inhaled corticosteroids (when those aren’t controlling asthma by themselves), i.e. Advair = salmeterol + fluticasone Symbicort = formoterol + budesonide

Answer 55

Block leukotrienes (released by cells, responsible for asthma symptoms) Singulair (Montelukast), Accolate used for seasonal allergies and maintenance tx of asthma

Answer 56

Used for severe persistent asthmaRecombinant DNA IgG that binds to free IgE in blood

Answer 57

N2O – appropriate AVOID narcotics, barbiturates Can use Vistaril/Atarax (hydroxyzine) antihistamine Be prepared to rescue the patient.

Answer 58

1. Discontinue tx 2. Remove everything from mouth 3. Position pt for comfort 4. Pulse-OX 5. Inhaler B2 6. Oxygen If spO2 \< 91% and struggling to breathe, Epinephrine 0.01 mg/kgcall 911

Answer 59

Yes. 13.4% Afr Am, 7% urban more common in low SES Childhood prevalence is 8.5% Most common chronic medical condition of childhood.

Answer 60

- Congenital abnormality of airway - Cystic fibrosis - Immunodeficiency - GERD - Foreign body - Malignancy

Answer 61

Beta2-agonist

Answer 62

1. Barbiturates (phenobarbital) 2. Narcotics (morphine & meperidine(=demerol)) stimulate histamine release leading to bronchospasm

Answer 63

1. Hydroxyzine (vistaril, antihistamine) 2. Benzodiazepines (potentiate GABA’s effects, diazepam (valium), lorazepam (ativan), midazolam (versed))

Answer 64

True. Also high palate and increased anterior facial height As well as…increased caries, candidiasis, erosion, decreased salivary flow, increased calculus & gingivitis

Answer 65

No, but can appear as congestive heart failure over time. ASD VSD PDA AVSD Increase in pulmonary blood flow at expense of systemic circulation

Answer 66

Yes. Less blood to lungs, pts appear cyanotic. Tetralogy of Fallot Highest risk of endocarditis

Answer 67

Anatomic narrowing: coarctation of aorta, aortic stenosis, pulmonic stenosis valvular, subvalvular, supravalvular Also appears as congestive \<3 failure over time

Answer 68

1. Dilated cardiomyopathy (limited ability of \<3 to contract due to abnormality of myocardium) could be from disease, medication 2. Hypertrophic cardiomyopathy – dysrhythmias, syncope, sudden death

Answer 69

Inability of heart to pump adequate amt of blood to systemic circulation at normal pressure to meet metabolic demands Managed medically, surgically, or combination: - remove extra fluid - improve cardiac function - improve tissue oxygenation - reduce demands on heart

Answer 70

1. Digoxin: increase contractility of \<3 2. Diuretics: decrease fluids 3. Warfarin: increases prothrombin time 4. ACE inhibitors: decrease blood pressure

Answer 71

Age 2-4 Repaired earlier if symptomatic. Some VSDs heal spontaneously

Answer 72

5 cases / 100,000 persons-years Less frequent in children

Answer 73

May appear as other infection - weakness, fatigue, rash, fever, anorexia, night sweats, arthralgia - 7–14 day typical incubation period, but may take up to 4 wks \*pedo patients more likely to acquire subacute rather than acute bacterial endocarditis

Answer 74

EXT 10-100% Perio sx 36-88% SRP 8-80% cleaning 40% RD placement 9-32% Toothbrushing/flossing 20-68% Toothpicks 20-40% CHEWING FOOD 7-51%

Answer 75

Give dose within 2 hrs

Answer 76

1. Delay dental procedure for 10 days to allow normal flora to re-establish 2. OR choose abx from another class (azithromycin or clindamycin)

Answer 77

1. Primary – platelet aggregation - aggregation caused by injury - release vWF & collagen fibers from endothelium - platelets adhere - vasoconstriction occurs 2. Secondary - coagulation cascade - Extrinsic pathway activated when injury exposes blood to TF - Intrinsic pathway produces F X - Common pathway generates thrombin

Answer 78

Initiation Amplification Propagation

Answer 79

Initiation: 1. TF binds VII 2. TF-VII activates IX + X 3. Xa + Va to produce small amt of thrombin

Answer 80

Amplification – occurs mainly in platelets 1. Uses thrombin from initiation activates V, VIII, IX (5, 8, 9) 2. Releases VIII from vWF 3. Ends with Va and VIIIa being bound to activated platelets (5a, 8a)

Answer 81

Propagation: 1. Activated VIII + IX form complex in platelet 2. This complex VIII-IX activates X 3. Xa binds with Va to form prothrombinase complex 4. Prothrombinase complex converts prothrombin to thrombin

Answer 82

Plasmin dissolves fibrin clotRegulated by antiplasmin and plasminogen activator inhibitors

Answer 83

Production of Vit-K dependent factors (II=prothrombin, VII, IX, X) effects can be reversed by administering fresh Vit K1 Onset and duration of action of single dose is 2-5 days

Answer 84

Pradaxa is a competitive, direct thrombin inhibitor - prevents clot development - prevents conversion of fibrinogen to fibrin

Answer 85

1. PT – EXTRINSIC – tests for defects in V, VII, X, thrombin, fibrinogen (normal is 1-18 seconds ) 2. PTT – INTRINSIC – tests for defects in V, VIII, X, XI, thrombin, fibrinogen 3. Platelet count (normal = 140,000 – 340,000) 4. Platelet function (PFA-100) – more accurate than bleeding time 5. Bleeding time – inaccurate in young children (1-6 min)

Answer 86

LOW 1. supragingival restorations or px 2. infiltration anesthesia INTERMEDIATE 1. Sub-g restoration 2. single EXT 3. RCT 4. Nerve blocks HIGH 1. Multiple EXTs 2. Perio sx 3. Gingival curettage

Answer 87

Von Willebrand disease 1% of populationdeficient or defective vWF (= plasma protein in endothelium) AUTOSOMAL DOMINANT INHERITANCE low levels of VIII (may have longer PTT) vW binds to VIII to protect it in blood prolonged bleeding time abnormal platelet function test VWD is a primary process defect (defect in platelet #/function) longer bleeding time, bleeding from superficial and deep cuts, petechiae, small multiple ecchymoses, spontaneous bleeding

Answer 88

DDAVP (for mild Hem A or vWD) \* synthetic analog of desmopressin. POTENT ANTIDIURETIC. Decrease fluid replacement rate. - causes rapid release of VIII & vWF (doubles levels) - peak effect 1 hr after administration, 30-45 min to take effect - IV or subQ - Stimate nasal spray (can repeat q24hrs, limit fluid intake 12-18 hrs) Amicar (aminocaproic acid) (prevent clot dissolution) - antifibrinolytic – inhibits plasminogen to plasmin - 2 hrs peak effect - IV or PO (50 mg/kg q6h until healed, 7 days) - may be given alone or with DDAVP - inhibits salivary enzyme fibrinolysis

Answer 89

Platelets Could be due to low production (aplastic anemia, BM cancer) increased breakdown of platelets (Idiopathic thrombocytopenic purpura or drug-induced immune thrombocytopenia) Symptoms: bruising, epistaxis, gingival bleeding, petechiae CBC – low platelets PTT, PT normal another primary process defect like VWD

Answer 90

Hemophilia A – 85% - Factor VIII (think ATE/EIGHT) Hemophilia B – 15% - Factor IV (think benign) Christmas 1/3 of cases are new mutations X-linked recessive inheritance secondary process defect (defect in coagulation pathway)

Answer 91

Hemophilia A \< 1% VIII activity severe (70%) 1-5% VIII activity moderate (15%) \> 5% VIII activity mild (15%) Hemophilia B \< 1% IX activity severe (50%) 1-5% IX activity moderate (30%) \> 5% IX activity mild (20%)

Answer 92

Normal platelet plug formation (no significant bleeding after superficial cuts, but significant bleeding after deep cuts; no petechiae, but large wide-spread ecchymoses; hematoma, hemathrosis, spontaneous bleeding)delayed formation of fibrin clot (friable/poorly formed clot) Types of bleeding: Deep bleeding into joints, muscles Increased bleeding from open wounds appears to stop, then restarts (problems with exfoliation of primary teeth)

Answer 93

1. Inhibitors – Ab block clotting factor activity (15% of severe hem A, 2.5% hem B pts) (tx w/high dose clotting factor, bypassing agents, induce immune tolerance) 2. Arthropathy - can occur without injury, chronic/acute pain, joint degeneration (knees, elbows, ankles most affected) 3. Bleeding into CNS or airway 4. HIV 5. Hepatitis

Answer 94

- Pressure - Sutures - Collagen sponge (increases platelet aggregation) - gelfoam (absorbs blood, forms matrix to organize clot) - Surgicel (resorbable oxidized cellulose) - bone wax (mechanical barrier to bleeding from bone) - Thrombin – converts fibrinogen to fibrin - electrocautery - epinephrine (re-bleeding likely to occur)

Answer 95

T. T. Acquired: pregnancy, surgery, immobilization Inherited: impaired function of protein C-anticoagulant system - Factor V Leiden : 5% in North America (increased prothrombin) Management: Acute: Heparin for several days, then Warfarin for 3-6 months Long-term: Anti-coagulant therapy – verify INR

Answer 96

Normal INR = 1 Therapeutic range 2 – 3 INR measures PT – Extrinsic pathway (F VII-TF → F X) heparin intrinsic // extrinsic warfarin

Answer 97

Autosomal recessive Most common genetic blood disorder 1 of 12 Afr Ams have SC Trait 1 of 600 Afr Ams have SC Anemia

Answer 98

ASA III Life expectancy ~40 yrs

Answer 99

- Systemic disease - Dehydration - Exposure to cold - Rigorous exercise - Infection - Acidosis - Stress

Answer 100

Shortened life span of sickled RBC’s 12 days Normal span 120 days

Answer 101

- Pale mucosa - Enamel hypoplasia - Dental or jaw pain from infarcts - increased incidence of osteomyelitis - delayed eruption - pulp calcification - “hair on end” lateral skull film - Class II - decreased trabeculation, thin inferior border, distinct radioopacities of mandible - decreased caries when taking abx “hair on end” also seen in neuroblastoma, Fe deficiency anemia, thalessemia

Answer 102

- AM appts, short - No contraindications to LA w/epi - N2O use okay but minimum 50% O2 - Acetaminophen for pain - ASA III - GA ➔ hydration, oxygenation, vascular stability, temp, avoid acidosis - treat infections vigorously – avoid elective surgery

Answer 103

Answer: a. achieves therapeutic levels minutes after adminNot always enough coverage for pts with inhibitors, half life of 8-12 hours, given IV, used in conjunction with desmopressin

Answer 104

Answer: C. Increases levels of factor IX desmopressin (DDAVP) causes a rise in Factor VIII and vWF

Answer 105

F. New terminology: Partial – simple or complex (40%) Generalized – convulsive or nonconvulsive (40%) Unclassified Status epilepticus – seizure lasting \> 30 mins

Answer 106

T. Originates from localized area of brainlocalized twitching, numbness/tinglingflashes of light, dissociation feelinglip smacking

Answer 107

T – partial – complex – localized area of brain F – 1-2 minute loss of consciousness experienced Prevalence of seizure types: Complex partial 36% gen. tonic clonic 23% simple partial 14%

Answer 108

Generalized. Formerly known as petit malInvolves entire brain, 10-30 sec LOChalf develop tonic-clonic seizures at puberty

Answer 109

“grand Mal” aura LOC leads to falling Tonic: muscle rigidity 10-20 secs Clonic: muscle contractions 2-5 minutes incontinence postictal period (deep sleep, HA, confusion) full recovery (3 hrs)

Answer 110

Seizure disorders Management of seizure disorders also includes ketogenic diet surgery vagal nerve stimulators

Answer 111

Seizure \> 5 minutes Or another seizure develops quickly since the last one status epilepticus

Answer 112

40% have parent with ADHD 35% have sibling with ADHD

Answer 113

- Occur at home and school - Be more severe than in other children same age - start before age 7 - continue for \>6 mo - difficult function in various settings More common in boys incidence: 3-5% school-age kids (more recent numbers 11%) Most commonly diagnosed behavioral disorder in childhood

Answer 114

- Inattentive - Hyperactivity - Impulsivity Can have various combinations of these 3 characteristics

Answer 115

ADHD – defiance In addition, depression (18%) and anxiety disorders (25%)

Answer 116

Non-amphetamine / CNS stimulant ADHD med

Answer 117

Yes. AKA dextro-amphetamine xerostomia, altered taste, bruxism \*Caution with Meperidine!\*

Answer 118

T. All ADHD meds have xerostomia as a side effect. This increases caries risk in 11-13 year olds Strattera – SNRI elevates BP – caution with vasoconstrictors Clonidine and Guanfacine (Intuniv) – anti-hypertensives also causes dysphagia potentiates CNS depressants – caution with sedative agents

Answer 119

True. Also increased bruxism and increased risk of dental trauma.

Answer 120

Spastic diplegia and quadriplegia (70-80%) stiff or rigid muscles, contractures and lack of control Other types include: Hemiparesis, athetoid or ataxic, hypotonic50% CP pts have mental developmental delay, 30-50% seizure disorder, 35% sensory deficits Oral findings: increased DMFS permanent teeth lower salivary rate, lower pH → increased caries enamel erosion poor gingival health delayed eruption malocclusion- AOB, class II tongue thrust bruxism increased gag reflex dysphagia, increased drooling, mouth breathing increased trauma

Answer 121

Males 3-4 times more likely to be affected. 6/1000 live births for ASD (New: 1/68)

Answer 122

No. Also, no words by 16 months, no smiling, eye contact, lack of imagination in play, compulsive behaviors, restricted interests and activities

Answer 123

Operant conditioning to teach specific skills

Answer 124

Hyperactivity – ritalin, concerta – enhance attention Repetitive = Prozac (fluoxetine) – antidepressants decrease compulsive beh & self-mutilation Aggressive – Tegretol (carbamazepine), Risperidone, Aripirozole (Abilify) – anti-aggression these have weight gain and sedation as side effects

Answer 125

1. Age (\<4, 4-7) 2. Reading (no vs. yes) 3. Toilet trained? 4. Concurrent medical diagnosis? 5. Expressive language used? \>2 of above risk factors strongly associated with uncooperative behavior

Answer 126

— Brachycephalic and flat occiput — Midface hypoplasia — Flat bridge of nose — Alantoaxial instability — Small ears — Inner epicanthal folds — Up-slanting palpebral fissures — Relative macroglossia, fissured tongue +/- benign migratory glossitis — Open mouth facies and protruding tongue — Constricted palate — Dry, fissured lips — Crowded Class III orthognathic tendency — Microdontia — Hypodontia — Delayed eruption and over-retained teeth — Enamel hypoplasia — Decreased parotid gland salivary flow — Increased risk for periodontal disease — Increased risk for obstructive sleep apnea (30-100%)† — Increased risk for Riga-Fede disease

Answer 127

Hypotonia intellectual disability increased incidence of cardiac defects (atrioventricular septal defect most common cardiac anomaly), hypothyroidism, ALL/AML, dementia, GI anomalies, Celiac diseaserisk of infx due to immunodeficiency palmoplantar keratosis, xerosis, short broad hands; single palmar crease (simian) A-A instability, bradycardia, airway challenges, and tendency for obesity are all factors to consider with sedation or GA

Answer 128

50-90% 36% of pts have primary dentition involved - not a lot of calc - bacteria same as other pts w/ intellectual disability - perio pathogens colonize earlier - abnormal capillary fragility - PMNs with chemotactic/phagocytic defects- normal # B-cells - T-cells dysfunctional – may have diminished ability to recognize and respond to specific antigens

Answer 129

Autosomal recessive chronic pulmonary dx, pancreatic insufficiency, high sweat electrolyte concentrations; chronic progressive dx life expectancy: median 37 years w/ treatment, 80% reach adulthood Most common in white people (1:3200) 2-4% of whites are carriers

Answer 130

Cystic Fibrosis Transmembrane Conductance Regulator -- decreased secretion of Cl- -- increased absorption of Na - exocrine glands produce thick sticky secretions → abnormal mucus formation affects lungs, intestine, upper airway and reproductive epithelium S/S: salty tasting skin failure to gain weight, abnormal bowel movementsrecurrent wheezing, cough, pneumonia; nasal polyps, sinusitis clubbing of fingers and toes pancreatic insufficiency

Answer 131

- Airway clearance techniques like chest physiotherapy, mechanical vest - exercise loosens mucus - aerosols (bronchodilators, mucolytics, decongestants, abx) - oral antibiotics

Answer 132

Pancreatic enzymes blocked in duct 90% of patients affected Malabsorption of fat and protein - poor weight gain - nutritional deficiencies - high fat stools - excess gas/abdominal pain Tx: increase caloric intake, pancreatic enzyme supplements, iron, Vit A, D, E, K (fat soluble)

Answer 133

Both are False. Increased enamel defects, erosion decreased caries salivary pH elevated partial xerostomia, parotid enlargement gingivitis and increased calculus mouth-breathing, malocclusion sinusitis, rhinitis – halitosis, referred pain decreased bone density (bisphosphonates) candidiasis, angular cheilitis (chronic antibx and steroids), traumatic lesions from coughing and devices

Answer 134

No. bc mucous accumulation overnight and increased coughing in morning. Short appt, later in the day, more upright chair position - Beware any sedative drugs that decrease respiration - chronic abx use may increase # opportunistic pathogens in airway – consult if oral infection - chronic steroid use – increase risk for candidal infection & delayed healing

Answer 135

Hair – sparse, fewer follicles, rough Eccrine – poor sweat glands (heat intolerance & hyperpyrexia with 30% mortality rate, can lead to seizures/neuro) Hypoplasia of salivary (xerostomia), lacrimal glands, mucus glands of UR/GI tracts -- dry skin, eyes, periorbital wrinkling and pigmentation, photophobia Dental – hypodontia to anodontia, microdontia (conical teeth), midface hypoplasia, under-developed alveolar ridges, decreased VDO Nail dystrophy (thin, brittle, deformed) Normal intelligence Ectodermal dysplasia is X-linked Recessive transmission, AD, and AR

Answer 136

Hypohidroticprevalence: 1:100,000 births

Answer 137

hypodontiaconical crownsdelayed eruptionpremature teethcleft lip/palate

Answer 138

70% have Type I, Insulin Dependent Diabetes Mellitus 1:400-600 children/adolescents Peak onset: 10-14 y; mini-peak onset: 4-6 y 30% type II

Answer 139

1. Polydipsia (increase in thirst and appetite) 2. Polyuria (frequent urination) 3. Weight loss\*, FTT \*polyphagia may not always occur in children

Answer 140

Absence or destruction of beta cells in pancreas leads to absolute deficiency of insulin Causes: genetics (HLA types DR3, DR4) environmental (e.g. Enteroviruses, being breastfed lowers risk), other – developmental (congenital absence), secondary damage (CF), chromosomal (Down, Turner, Kleinfelter, Prader-Willi syndromes) Tx: insulin injections, insulin pump, diet, pancreatic islet cell transplant (experimental)

Answer 141

Symptoms + blood glucose levels measured: fasting glucose is ≥ 120 mg/dL, random ≥ 200 mg/dL HbA1C is elevated when blood glucose is high. Good metabolic control then fewer periodontal implications Normal HbA1C = 7-9

Answer 142

10 – 15% because of - increased rxn to plaque - increased severity/destruction 3x more perio disease than general population - impaired immune fn (PMN defects) - decreased vascular efficiency

Answer 143

Fasting : 120 mg/dL Random: \< 200 mg/dL HbA1c (normal is 7-9%)

Answer 144

30% with increasing incidence. Body doesn’t use insulin properly (insulin resistance with relative deficiency) Characterized by hyperglycemia, vascular disease, and neuropathy

Answer 145

Type II diabetes Cutaneous marker of insulin resistance (especially on neck) Leathery or velvety, thickened to papillary brownish patches in flexural sites, axilla, neck, groin, fingers, lips

Answer 146

Metformin – reduces glucose production in liver and increases insulin sensitivity (drug of choice for children \>10 yrs) oral hypoglycemics injected insulin Diet management and exercise crucial Eat foods wit low to medium Glycemic Indexfat and protein have minimal effect on blood glucose

Answer 147

True increased gingivitis, periodontitis prolonged infxs, candidiasis, xerostomia, sialadenosis delayed wound healing altered sensations: burning, taste odontalgia (microangiopathy) OSA (DM II) Lichen planus, acetone breath, ?benign migratory glossitis (DM I) AAP recommends annual periodontal eval starting at age 6!

Answer 148

False. The level of diabetic control is more important than plaque control

Answer 149

AM appts! Short appts Normal insulin dose + normal meal day of appt Ask about hx of hypoglycemia Bring glucometer to appt, check pre-op (if low, consider carbohydrate before starting case) Have source of glucose available Maximize prevention (perio), manage infections aggressively (consider antibiotics), discuss diet/alcohol/tobacco effects on disease control

Answer 150

ADH deficiencyantidiuretic hormone deficiency

Answer 151

Mild: hunger, weakness, sweating, increased pulse, pale moist skin, confusion, sweating Moderate: incoherence, uncooperativeness, belligerence Severe: LOC, tonic/clonic mvmts, hypotension, hypothermia Hypoglycemia management: Administer glucose (tabs, juice, honey, hard candy, frosting); retest blood sugar; repeat in 15 min if not improved. Insulin shock (\<40 mg/dL): administer dextrose sublingual, IV or glucagon IM

Answer 152

1. Symptoms and tissue damage: esophagitis, erosive GERD 2. symptoms and no tissue damage: non-erosive GERD

Answer 153

Lower esophageal sphincter

Answer 154

Eosinophilic esophagitis – co-exists with atopic conditions like eczema, asthma, rhinitis

Answer 155

difficult of painful swallowing gingival inflammation oral erosions and ulcers hypersalivation palatal erythema, petechiae coated or discolored tongue halitosis enamel erosion (mx palatal surfaces first then occlusal surfaces) higher salivary microbial colonization increases risk for caries! Use fluoride varnish, topical f-, calcium phosphate paste for sensitivity; do not brush teeth after vomiting

Answer 156

Antacids (neutralize stomach pH) Foaming agents (coat stomach contents) H2 receptor antagonists – inhibit acid production proton pump inhibitors – impede acid production (better drug)

Answer 157

Yes. Viral load \< sensitivity of test

Answer 158

Ck labs Neutropenia in 50% children with AIDS, 10% asymptomatic children Anemia: 20-80% Thrombocytopenia infectious diseases common immunologic disorders common oral side effects of HIV drugs (xerostomia, vomiting, taste alterations, sucrose content) over-retained primary teeth, delayed eruption dental caries and enamel hypoplasia symptomatic orofacial lesions referred pain: sinusitis, otitis media, neuropathies increased risk for Vit D deficiency

Answer 159

Oral lesions are common in pediatric HIV pts (up to 80%). Oral lesions are associated with decreased immunity and may signal advancing disease CD4 depletion common with presence of oral infections

Answer 160

Every 3 mo Prevention is critical — Regular recalls, every 3 months — Chlorhexidine may be helpful, if oral hygiene is poor — Fluoride supplementation as indicated Caretaker may be sick and may not be as involved in child’s oral health Poor access to medical and dental care may be complicating factor

Answer 161

Zovirax (acyclovir) 15 mg/kg 5x/day Famvir and Valtrex are not approved for pediatric use

Answer 162

EBV lateral border of tongue, white plaques, asymptomatic/burning sensation, lesion waxes and wanes

Answer 163

EBV, HHV-8, HPV Kaposi’s – HHV-8 lymphoma – EBV, HHV-8

Answer 164

1. Leukemia 30% leading cause of cancer death \<1 yo and 10-19 yo 2. CNS Tumors 17% leading cause of cancer death ages 1-5, most common solid tumor of childhood 3. Neuroblastoma 7% most common malignancy in the first year of life 4. Non-Hodgkin lymphoma and Wilms tumor 6% 5. Hodgkin disease 5% 6. Rhabdomyosarcoma 3% most common soft tissue sarcoma of childhood

Answer 165

1. Sarcomas esp rhabdomyosarcoma 76% 2. Carcinoma 18% 3. Esthesioneuroblastoma 6% s/s facial swelling and pain, orbital displacement

Answer 166

No. 75% of patients will be long-term survivors. There will be late effects of therapy however, like - problems with organ function, growth & development - problems with neurocognitive fn and academic achievement - increased risk for 2nd cancer - challenges with career opportunities - access to health care/insurance (should no longer be with ACA!)

Answer 167

AD, 1:3000 births, 50% spontaneous mutation Stigmata: neurofibromas, café au lait macules, freckling in axillary or inguinal regions, Lisch nodules, CNS disorders **Oral lesions in 70%: enlarged fungiform papilla 50%, oral fibromas 25%, widened mandibular canal 25% and foramen** Tx: excision if cosmetic concern or obstruction Malignant transformation rate: 5% for large lesions

Answer 168

Neurofibromatosis McCune-Albright syndrome (endocrine disease, polyostotic fibrous dysplasia, precocious puberty in F) Tuberous sclerosis (hamartomatous skin nodules, seizures, bone lesions, enamel hypoplasia, MR) Fanconi anemia

Answer 169

ALL \>80% of leukemias leukemias represent 30% of all cancers 5 yr survival 90% Highest risk in white males AML is 19% of leukemias 5 yr survival 50%

Answer 170

AML ALL: petechiae of skin, mucosa (50%), NUG, cellulitis, persistent ulcers, gingival bleeding and mobile teeth

Answer 171

T. Need lifelong immunosuppressive therapy w/any transplant Kidney transplant indicated w/end stage renal disease. 98% 1 yr, 94% 5 yr survivals Acute renal failure: blood loss from trauma, dehydration, poisons/meds, disease, necrosis, obstruction of UT Chronic renal failure: cystic dx, anatomic abnormalities, glomerulonephritis

Answer 172

Peritoneal dialysis (dialysate fills abdomen, capillaries move waste into dialysate in abdomen, dialysate is drained/discarded) Hemodialysis (takes blood out of body and filters it)

Answer 173

Renal osteodystrophy - loss of lamina dura - demineralized bone, loss of trabeculation (ground glass) - localized radiolucent lesions (giant cell lesions) - fracture or tumors from secondary hyperparathyroidism

Answer 174

F. - Pallor of oral mucosa, gingival bleeding, increased calculus - malodor, metallic taste, xerostomia, uremic stomatitis - Increased salivary pH, reduced caries rate - Enamel hypoplasia/discoloration, tooth erosion - delayed tooth eruption, eruption of rootless teeth - glossitis - enlarged major salivary glands - Petechiae and ecchymoses - soft tissue calcification, PDL widening, pulp obliteration - renal osteodystrophy - loss of lamina dura, radiolucent giant cell lesions, lytic areas of bone, ground glass appearance (decreased trabeculation), bone demin - anemia, thrombocytopenia, defective platelet fn - increased infection

Answer 175

1. Cholestatic liver disease (biliary atresia most common cause) 2. Fulminant hepatic disease (drugs, acetaminophen, toxin exposure) 3. Metabolic liver disease 4. Liver tumors (hepatoblastoma)

Answer 176

F. Green stain in enamel (biliverdin) Yellow discoloration of oral soft tissues (jaundice) Enamel hypoplasia due to metabolic disturbance petechiae, ecchymoses Delayed eruption Enlarged pulp chambers fetor hepaticus: sweet, musty breath mucosal bleeding

Answer 177

- Altered drug metabolism - Similar considerations to pts receiving chemo - may be infectious if HBV or HCV is cause - malnutrition - frequent recall - immunosuppression - bleeding considerations - frequent recall

Answer 178

F. It is one of the most successful. 600 performed annually 12,000 total pediatric liver transplants 91% survival 1 yr, 87% 5 yr

Answer 179

True. IL-6 can identify individuals at risk for organ rejection (higher IL-6 w/ chronic perio) Other dental mgt considerations of solid organ transplant: risk for gingival overgrowth – specific to this group reinforce oral prevention program monitor and treat opportunistic infections caution w/ antibiotics – nephrotoxicity manage xerostomia risk of “de novo” dysplasias and malignancies (epithelial dysplasia, NHL, SCC, BCC)

Answer 180

- Increased risk for infection - Change in appearance - Cushingoid appearance - acne - obesity - hirsutism - increased cancer risk - hypertension - osteoporosis

Answer 181

Cyclosporine - use in peds pts decreased to \<15% in ’96) - gingival enlargement in 25% Tacrolimus - candidiasis, ulcers Azathioprine - thrombocytopenia, leukopenia, anemia, skin cancer Steroids - insomnia, nervousness, infections Calcium Channel Blocker: Nifedipine - may be rx’d to minimize nephrotoxic/HTN effects of cyclosporine and tacrolimus - gingival overgrowth ``` Mycophenolate Mofetil (MMF) - leukopenia, GI problems, myelosuppression ``` Sirolimus & Everolimus - delayed wound healing, bone marrow suppression, oral ulcers

Answer 182

- Nightly snoring - Disturbed sleep - Daytime neurobehavioral problems

Answer 183

F. No cure or effective treatment Pseudohypertrophic MD (Duchenne) = most common neuromuscular disease of childhood X-linked recessive (all males affected)

Answer 184

Can’t walk by 12. Universally fatal – death prior to age 30 from respiratory or cardiac complications 25% demonstrate MR elevation of creatine kinase by 50-100 times related to muscle damage

Answer 185

Neural tube defect Etiology can be: - folic acid deficiency - maternal insulin-dependent diabetes - maternal obesity, hyperthermia, antiepileptic drugs 1,500 infants/yr in US

Answer 186

- Latex allergy precautions - seizures, obesity - Decreased bone density - MR? - fear of choking w/reclining – consider treatment inclined slightly and more upright as supine position may be difficult - abx px for VV or VA shunt?

Answer 187

Incidence: 1 in 500 children Symptoms: rapid increase in head circumference in infants, vomiting, seizures, downward casting of eyes (“sunsetting”), sleepiness, irritability, blurred vision, inability to balance

Answer 188

F. Increased salivary bacteria - aspiration pneumonia

Answer 189

Suicide, depression, substance abuse, cardiac arrythmias & arrest, esophagitis/pancreatitis, seizures, aspiration, impaired renal function.

Answer 190

10-15% have unilateral or bilateral parotid gland enlargement and dry mouth

Answer 191

Calluses on the knuckles or back of the hand due to repeated self-induced vomiting (from knuckles contacting incisors)

Answer 192

Binge eating disorder recurrent episodes of binge eating in the absence of the regular use of inappropriate compensatory behaviors such as purging, fasting, excessive exercise

Answer 193

SSRI (selective serotonin reuptake inhibitors) - cognitive behavior therapy - interpersonal therapy for adolescents

Answer 194

T. 40% within 2 years 72% within 5 years

Answer 195

ADHD meds (stimulants or non-stimulants) Meds for depression, phobias, anxiety, eating disorders, OCD, personality disorder, PTDS like - SSRI (fluoxetine, paroxetine, sertraline, fluvoxamine, citalopram) - tricyclic antidepressants (second choice after SSRIs) (Imipramine, nortriptyline, desipramine, amitriptyline) \*xerostomia

Answer 196

a. Smooth surfaced, expansile lesions b. More common in the maxilla c. Most likely to be present at birth d. Avascular e. Poorly circumscribed radiolucencies “floating teeth” Melanotic neuroectodermal tumor of infancy

Answer 197

b. Hearing loss Sturge-Weber: - Vascular malformations of face and brain - Congenital port-wine stain follows trigeminal nerve - Ipsilateral facial angiomatosis - There is ocular involvement, MR, hemiplegia (CNV involved), seizures - Oral: hypervascularity, pyogenic granulomas, gingival overgrowth, alveolar bone loss, bleeding; supernumerary teeth; overgrowth of bony maxilla, enamel hypoplasia, accelerated eruption, talon cusp

Answer 198

Dendritic cell and macrophage (White blood cells) proliferation and infiltration into tissues

Answer 199

1. Unifocal – (formerly known as Eosinophilic Granuloma misnomer) no extraskeletal involvement – excellent prognosis 2. Multifocal – mostly seen in children age 2-10, fever, bone lesions, diffuse eruptions. Good prognosis. Hand-Schuller-Christian triad 1. diabetes insipidus, 2. exophthalmos, and 3. lytic bone lesions 3. Multifocal multisystem – aka Letterer-Siwe disease. Children ≤ 2 yo, poorest prognosis

Answer 200

“floating teeth”\*\*\* caused by bone lesions. Punched out lesions of skull 10% show oral involvement (which is the usual initial infiltration area) “floating teeth” can also be seen in melanotic neuroectodermal tumor of infancy, traumatic bone cyst.

Answer 201

- neoplasm - immunodeficiencies - metabolic defects Criteria: pruritus, pattern of skin involvement (young children – extensor surfaces, cheeks, forehead, neck; older children – flexor surfaces), family hx, young age of onset, elevated serum IgE and eosinophil counts

Answer 202

Atopic dermatitis 90% present by age 5

Answer 203

Food allergen

Answer 204

- High dose corticosteroids – consult pmd

Answer 205

Hives. Definition: superficial dermal layers affected by pruritic, erythematous raised lesions (blanch with pressure)

Answer 206

DEEP swelling that affects the face, extremities, genitalia, occasionally tongue and larynx (under the skin, not on surface)

Answer 207

40% hives with underlying swelling

Answer 208

Submucous or subcutaneous edema - non-pitting - tensely swollen - can be painful? - NOT erythematous/warm/pruritic Autosomal dominant deficiency in functional C1 esterase inhibitor (complement system)

Answer 209

Trauma medical/dental procedure stress menstruation infections oral contraceptives medications foods, plants heat, cold, sun, pressure, exercise

Answer 210

- Lips - Eyelids - Tongue - Extremities - Genitalia - Oropharyngeal/laryngeal is most concerning (bc of airway obstruction potential)

Answer 211

NO. Acute attack: Purified C1-esterase inhibitor (Berinert), recombinant protein inhibitor of kallikerin, ecallantide(Kalbitor) Epi and antihistamines are not useful Flaitz lecture: could use benadryl for mild cases; severe cases – IM Epi, IV steroids and antihistamines; C1-INH deficiency – Danazol, stanozolol for prevention

Answer 212

IgE-mediated in 6-8% of kids \< 3 yo. 2% prevalence overall

Answer 213

1. Eggs **2. Peanuts** 3. cow’s milk 4. Soy **5. Tree nuts** **6. Fish 7. shellfish** 8. wheat ones in bold persist after childhood

Answer 214

5–10% of general population higher (up to 17% of health care workers)

Answer 215

F. Caused by glove wearing/handwashing/sanitizers/other chemicals Non-immunological mediated dry/itchy/irritated skin

Answer 216

Yes. It is t-cell mediated rash/redness/itching 24-48 hrs after contact

Answer 217

No – rarely anaphylaxis. It can progress to urticaria, allergic rhinitis, sneezing, wheezing, coughing, difficulty breathing. IgE-mediated swelling, redness, itching

Answer 218

- Ppl who have undergone multiple surgical procedures - spina bifida 18-73% - first surgery before age 1 - spinal cord trauma, urogenital/GI malformations, neurogenic bladder, hydrocephalus w/ VP shunts - atopic individuals - occupational exposure - food allergies like avocados, bananas, chestnuts, figs,kiwis, turnips, tomatoes, potatoes - fruit allergy 11% risk of concurrent latex allergy

Answer 219

_1. Juvenile (Idiopathic) Arthritis_ - consider that person is in pain and may have TMD _2. Vasculitides_ - **Wegener granulomatosis** (involves medium and small arteries, venules, arterioles, extremely rare in children) - **Bechet** (very rare in children, *oral apthous ulcers*, ocular disease, large vessel vasculitis 33%, small vessel common) _3. Systemic Lupus Erythematosus_ - ***_most common presentation in childhood_***: fever, malaise, failure to thrive - *malar rash only present in 1/3 patients* - Assess kidney function - increased susceptibility to infection - depletion of antibodies - secondary heart damage - **Sjogren syndrome** can be secondary complication

Answer 220

Ellis van creveld “6 fingered dwarfism” – short distal extremities, short stature, polydactyly 40-50% have cardiac defects chondrodysplasia hidrotic ectodermal dysplasia ***neonatal teeth, multiple frenae hypodontia, enamel hypoplasia, microdontia may have delayed exfoliation/eruption or accelerated eruption BOTH! \**** \*Other condition as having both delayed exfoliation and accelerated eruption is OI.

Answer 221

Van der Woude Paramedian lip pits: persistence of lateral sulci on embryonic arch congenital invaginations of lower lip

Answer 222

HPV 13, 32 =Multifocal Epithelial Hyperplasia proliferation of oral squamous epithelium seen in certain ethnic groups (Mexicans, Navajo, South Americans), seen in children characterized by multiple raised papules on lower lip, buccal mucosa, tongue

Answer 223

Goldenhaar syndrome aka Hemifacial Microsomia

Answer 224

50% etiology unknown unilateral microtia, macrostomia, failure of formation of mandibular ramus and condyle 1st and 2nd arch Tx: distraction osteogenesis, orthognathic surgery

Answer 225

Answer: A. Achondroplasia- short limb dwarfism, caudal narrowing of spinal canal, 1 in 20,000 (most common form of dwarfism), hypotonia (caution sedation), trident hands= short metacarpals & phalanges, midface hypoplasia, frontal bossing, hypodontia, delayed primary exfoliation/perm. Eruption Chondroectodermal dysplasia (Ellis-van Creveld)- hypodontia, short distal extremeties, polydactyly, nail hypoplasia, autosomal recessive, neonatal teeth, enamel hypoplasia, conical crowns, microdontia, multiple frena, 50% have cardiac defect usu ASD, prenatal onset of short stature (form of dwarfism), seen in Amish populations Hallerman-Streiff syndrome- hypodontia or hyperdontia, mandibular hypoplasia, high palatal vault, premature/natal teeth, delayed primary exfoliation, malar hypoplasia, microphthalmia, small pinched nose, hypotrichosis (less than normal amount of hair), “wormian” bones (abnormal intrasutural bones), narrow upper airway Hypopituitarism- growth hormone deficiency, small, well-proportioned unlike achondroplastic dwarf; dental findings- hypodontia, delayed tooth eruption, smaller mandible, microdontia, shortened roots, crowding/malocclusion due to small dental arches, hypofunction of salivary glands, higher caries/perio risk Morquio syndrome- inherited disease of metabolism in which body cannot break down glycosaminoglycans (formerly called mucopolysaccharides); autosomal recessive, abnormal bone development including spine, heart problems, loss of nerve function below the neck; normal cognitively

Answer 226

Fetal alcohol syndrome

Answer 227

Answer: D. Ehlers-Danlos syndrome (collagen defects) Other conditions with dentin abnormalities: Vit D resistant rickets, hypoparathyroidism, pseudohypoparathyroidism, Albright’s hereditary osteodystrophy Apert syndrome- supernumerary teeth Cleidocranial dysplasia- supernumerary teeth Down syndrome- supernumerary teeth, taurodontism Gorlin-

Answer 228

Hypophosphatemic rickets X-linked dominant; 1:20k births Mutation in zinc metalloproteinase gene - decreased resorption of phosphate in renal tubules - short bowed extremities - dental: large pulps, high pulpal horns, hypomindentin, abscesses, enamel may be spared Tx: calcitriol, phosphate

Answer 229

AD for classic forms (80%) - Flaitz; handbook says x-linked Stigmata: hyperelastic skin, scarring, hypermobility of joints, subluxation of TMJ, bruise easily delayed healing Oral: periodontitis, Gorlin sign, bleeding, pulp stones, +/- short roots, prominent cusp tips, hypoplastic enamel, dentin abnormalities

Answer 230

- Pitted teeth - Enamel hypomineralization - Taurodontism (w/ PARLs) - delayed eruption - dental hypoplasia - sclerotic dense bone - dolicocephaly with frontal bossing (AI + taurodontism + nail and hair defects)

Answer 231

Aperts Cleidocranial dysplasia Gardner Crouzon\* Sturge-weber Orofacialdigital syndrome\* Hallerman-Strieff\* cleft lip/palate\* Down syndrome \*also hypodontia

Answer 232

Seckel Williams Rieger achondroplasia chondroectodermal dysplasia ectodermal dysplasia Crouzon\* cleft lip/palate\* incontinentia pigmenti orodigitalfacial syndrome\* Hallerman-Strieff\* \*also hyperdontia

Answer 233

Crouzon syndrome Down syndrome chondroectodermal dysplasia ectodermal dysplasia hemifacial microsomia oligodontia

Answer 234

Maxillary hypoplasia +/- cleft palate small maxillary sinuses supernumerary teeth _delayed dental development_ and eruption/delayed primary exfoliation/failure of permanent tooth to erupt enamel hypoplasia deficient cellular cementum brachycephaly, frontal and parietal bossing, depressed nasal bridge, Wormian bones CBFA1 (RUNX2) gene defect 1:100k prevalence inherited bone disorder, AD w/ 40% spontaneous mutation affects craniofacial, clavicles, dental Tx: multidisciplinary care

Answer 235

Type 1: most common X-linked dominant syndactyly, clinodactyly, extra digit intellectual disability hypoplastic alar cartilage hypotrichosis _Oral anomalies:_ - hamartoma/choristoma on tongue - multiple frenae - bifid, multilobed tongue - hypodontia (lower 2s) or hyperdontia (upper 3s) - cleft of palate, alveolus - “bumpy jaw” - brachycephaly

Answer 236

Lesch-Nyhan Syndrome X-linked recessive absence of hypoxanthine-guanine

Answer 237

Answer: E. Sturge-Weber and dens evaginatus- talon cusp (lobodontia), also supernumerary teeth; other syndromes with talon cusp- Mohr, Rubinstein-Taybi, orofacial digital II syndrome Crouzon- hyper/hypodontia, inverted V-shaped palate, V-shaped arch, maxillary hypoplasia, brachy, microdontia or macrodontia, exopthalmia, no hand anomalies, hypertelorism Hallerman-Streif- hyper/hypodontia, delayed exfoliation; Ichthyosis- delayed primary exfoliation, ankylosis, dilaceration; Pseudohypoparathyroidsim- dentin abnormalities (e.g. dentin dysplasia), enlarged pulp, small crowns, short blunted roots, pitted enamel, enamel hypoplasia, enamel attrition, delayed eruption, malformed or impacted teeth, partial anodontia

Answer 238

Answer: B. Ellis-van Creveld; also, pachyonychia congenita Other syndromes with natal teeth are Histiocytosis X and Pfeiffer

Answer 239

Hypodontia growth deficiency beginning at birth microcephaly, facial hypoplasia MR AR

Answer 240

Answer: Prothrombin time (PT) Pneumonic- PET (extrinsic, PT) and PITT (intrinsic PTT)

Answer 241

Supernumerary teeth delayed eruption multiple osteomas of the jaw also: epidermoid and dermoid cysts polyposis large intestines w/ high malignant potential AD

Answer 242

Answer: Treacher Collins syndrome malar and mandibular hypoplasia, downsloping palpebral fissures, malformed ears with associated hearing loss, and abnormalities of the eye including lower lid coloboma and absent lashes, cleft palate 30%. Airway anomalies may occur, causing life-threatening problems in the newborn period. Intelligence is generally normal Gene: TCOF1 increase with paternal age defect of 1st and 2nd branchial arches Tx: distraction osteogenesis

Answer 243

Apert Syndrome = acrocephalosyndactyly AD, increase with paternal age ocular proptosis, hypertelorism, parrot nose midface hypoplasia; narrow, high palate; cleft palate 75% or bifid uvula; delayed or ectopic eruption; supernumerary teeth; shovel-shaped incisors; V-shaped maxilla; class III with AOB; crowded dentition Tx: craniofacial surgery

Answer 244

Answer: has a genetic predisposition; neurobehavioral disorder, increased caries risk, symptoms present prior to age 7, treated with stimulant medications

Answer 245

Answer: Paternal age; folic acid deficiency most common, also maternal epilepsy medication use

Answer 246

Arnold-Chiari malformation \*for patients with this, consider tx in a more upright position (pts may feel discomfort and/or choking on reclining)

Answer 247

Apert Syndrome

Answer 248

Answer: c. Leukemia (30%), CNS 17%, Neuroblastoma 7%, Non-Hodgkin lymphoma 6%, Wilms tumor 6%

Answer 249

Answer: d. 120 days; sickled RBC survives 12 days

Answer 250

Answer: B. Platelets 35,000 (normal 150-340K), ANC \>1000 ok, RBC 4-6 mil normal, lymph 25-30% normal

Answer 251

Seizure disorder

Answer 252

Moebius syndrome ☹ baby

Answer 253

Coloboma of the eye Heart anomaly (TOF) Atresia, choanal Retardation (growth/cognition) Genital anomalies Ear anomalies

Answer 254

Vertebral defects Anal atresia Tracheo-esophageal fistula Radial and renal dysplasia Single umbilical artery

Answer 255

Narrowing of eye opening (blepharophimosis) lateral displacement of inner canthi ptosis (droopy eyelids) cupping of ears hypotonia premature ovarian failure in type I AD normal intelligence

Answer 256

Triad of: micrognathia/mandibular deficiency glossoptosis cleft palate (U-shaped) 15-25% w/ heart disease

Answer 257

Aka Cryptophthalmos Syndrome cryptophthalmos (fusion of eyelids) defect of auricle cleft palate mental deficiency 50% renal defects laryngeal stenosis partial syndactyly 20% still born, 20% die w/I 1st year AUTOSOMAL RECESSIVE

Answer 258

Aka Melnick-Fraser Syndrome branchial arch II anomalies – branchial cleft cysts hearing loss, preauricular pits/tags, malformed pinnae renal abnormalities long, narrow face; constricted palate; deep bite 1:40,000 AD

Answer 259

Branchio-oculo-facial syndrome

Answer 260

Waardenburg Syndrome congenital hearing loss changes in pigmentation of hair, skin eyes (e.g. different colored eyes, pale blue eyes, white forelock of hair, premature graying) broad mandible AD

Answer 261

Aka Cervico-oculo-acoustic syndrome asymmetry with short neck, low hairline fusion of two or more cervical vertebrae (Klippel-feilanomaly) abnormal eye movements – Duane anomaly (limitation of horizontal eye movement/abducens paralysis) congenital hearing impairment, preauricular skin tags/pits normal intelligence affects females primarily

Answer 262

Multi-organ disorder results in cell injury affects 1:4000 children increases susceptibility to MH

Answer 263

False – most are AR Hurler, Scheie, Sanfilippo-A,B, Morquio-A,B and Maroteaux-Lamy are AR; Hunter is X-linked recessive Oral findings: macroglossia, gingival hyperplasia (GAGs in gingival tissue), tooth spacing, enlarged dental follicle, unerupted teeth (delayed eruption), enamel hypoplasia

Answer 264

Medullary carcinoma of thyroid, pheochromocytomas (neuroendocrine tumor of the medulla of the adrenal gland) – hypertensive crisis Marfanoid body, narrow face, full lips, mucosal neuromas Oral findings: multiple, soft papules or nodules; pink; smooth surface; nontender Site: eyes, lips, tongue, buccal mucosa, gingiva Tx: excision of large mucosa neuromas AD

Answer 265

Beckwith-Wiedemann Syndrome

Answer 266

Prader-Willi syndrome Chromosome 15 deletion inherited from mother is Angelman syndrome (“puppet-like” gait, bouts of laughter, open-mouth facies, seizures, MR)